Skip to main content

Current Management of Medulloblastoma

  • Chapter
Current Techniques in Neurosurgery
  • 148 Accesses

Medulloblastoma is the most common posterior fossa tumor of childhood. Affected patients typically present with rapidly progressive headache, nausea, and vomiting from obstructive hydrocephalus. Medulloblastomas are inherently invasive lesions and show a high frequency of dissemination at diagnosis. Thus, although surgery plays an essential role in reducing the tumor burden and in opening the ventricular system and although it has an impact on prognosis, multi modal adjuvant therapy is essential to treat what is in reality an invasive, potentially multicentric disease process. During the past 25 years, advances in the surgical and adjuvant management have substantially improved the outlook for children with these tumors, making this one of the true “success stories” of pediatric neuro-oncologyi however, there is still substantial room for further progress, particularly in children with extensive postoperative residual disease and in those with dissemination. Ongoing studies are exploring ways of improving further the survival rate in these “high-risk” patients and reducing the treatment-related long-term morbidity in “standard-risk” patients. This chapter reviews recent advances in the molecular biology, diagnosis, and treatment of these tumors.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 74.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Pollack IF: Brain tumors in children.N Engl J Med1994, 331:1500–1507.

    Article  PubMed  CAS  Google Scholar 

  2. Roberts RO, Lynch CF, Jones MP,et al.: Medulloblastoma: A population-based study of 532 cases.J Neuropathol Exp Neurol1991,50:134–144.

    Article  PubMed  CAS  Google Scholar 

  3. Maleci A, Cervoni L, Delfini R: Medulloblastoma in children and adults: A comparative study.Acta Neurochir1992, 119:62–67.

    Article  CAS  Google Scholar 

  4. Haie-Meder C, Song PY: Medulloblastoma: Differences in adults and children.Int J Radiat Oncol Biol Phys1995,32:1255–1257.

    Article  CAS  Google Scholar 

  5. Bunin GR, Kuijten RR, Buckley JD,et al.: Relation between maternal diet and subsequent primitive neuroectodermal brain tumors in young children.N Engl J Med1993, 329:536–541.

    Article  PubMed  CAS  Google Scholar 

  6. Evans DGR, Farndon PA, Burnell LD,et al.: The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma.Br J Cancer1991, 64:959–961.

    Article  PubMed  CAS  Google Scholar 

  7. Johnson RL, Rothman AL, Xie J: Human homolog of patched, a candidate gene for the basal cell nevus synrome.Science1996, 272:1668–1671.

    Article  PubMed  CAS  Google Scholar 

  8. Raffel C, Jenkins RB, Frederick L,et al.: Sporadic medulloblastomas contain PTCH mutations.Cancer Res1997,57:842–845.

    PubMed  CAS  Google Scholar 

  9. Mori T, Nagase H, Hori A,et al.: Germ-line and somatic mutations of the APC gene in patients with Turcot syndrome and analysis of APC mutations in brain tumors.Genes Chromosomes Cancer1994,9:168–172.

    Article  PubMed  CAS  Google Scholar 

  10. Hamilton SR, Lui B, Parsons RE,et al.: The molecular basis of Turcot's syndrome.N Engl J Med1995,332: 839–847.

    Article  PubMed  CAS  Google Scholar 

  11. Yong WH, Raffel C, von Deimling A,et al.: The APC gene in Turcot's syndrome [letter].N Engl J Med1995, 333:524.

    PubMed  CAS  Google Scholar 

  12. Malkin D, Li FP, Strong LC,et al.: Gennline p53 mutations in a familial syndrome of breast cancer, sarcomas, and other neoplasms.Science1990, 250:1222–1228.

    Article  Google Scholar 

  13. Bigner SH, Mark J, Friedman HS,et al.: Structural chromosoinal abnormalities in human medulloblastoma.Cancer Genet Cytogenet1988, 30:91–101.

    Article  PubMed  CAS  Google Scholar 

  14. Biegel JA, Rorke LB, Packer RJ,et al.: Isochromosome 17q in primitive neuroectodermal tumors of the central nervous system.Genes Chromosomes Cancer1989, 1:139–147.

    Article  PubMed  CAS  Google Scholar 

  15. Raffel C, Thomas GA, Tishler DM,et al.: Absence of p53 mutations in childhood central nervous system primitive neuroectodermal tumors.Neurosurgery1993,33:301–306.

    Article  PubMed  CAS  Google Scholar 

  16. Saylors RL, Sidransky D, Friedman HS: Infrequent p53 mutations in medulloblastoma.Cancer Res1991,51:4721–4723.

    PubMed  Google Scholar 

  17. Cogen PH, McDonald JD: Tumor suppressor genes and medulloblastoma.J Neurooncol1996, 29:103–112.

    Article  PubMed  CAS  Google Scholar 

  18. Griffin CA, Hawkins AL, Packer RJ,et al.: Chromosome abnormalities in pediatric brain tumors.Cancer Res1988,48: 175–180.

    PubMed  CAS  Google Scholar 

  19. Wasson JC, Saylors RL III, Zeltzer P,et al.: Oncogene amplification in pediatric brain tumors.Cancer Res1990,50:2987–2990.

    PubMed  CAS  Google Scholar 

  20. Segal RA, Goumnerova LC, Kwon YK,et al.: Expression of the neurotrophin receptor TrkC is linked to a favorable outcome in medulloblastoma.Proc Natl Acad Sci USA1994,91:12867–12871.

    Article  PubMed  CAS  Google Scholar 

  21. Provias JP, Becker LE: Cellular and molecular pathology of medulloblastoma.J Neurooncol1996; 29: 35–43.

    Article  PubMed  CAS  Google Scholar 

  22. Packer RJ, Sutton LN, Rorke LB,et al.: Prognostic importance of cellular differentiation in medulloblastoma of childhood.J Neurosurg1984, 61:296–301.

    Article  PubMed  CAS  Google Scholar 

  23. GoldbergStern H, Gadoth N, Stern S,et al.: The prognostic significance of glial fibrillary acidic protein staining in medulloblastoma.Cancer1991,68:568–573.

    Article  PubMed  CAS  Google Scholar 

  24. Maraziotis T, Perentes E, Karamitopoulou E,et al. Neuron-associated class III flitubulin isotope, retinal Santigen, synaptbphysin, and glial fibrillary acidic protein in human medulloblastomas: A clinicopathological analysis of 36 cases.Acta Neuropathol (Berl)1992, 84:355–363. 25. Bailey P, Cuship.g H: Medulloblastoma cerebelli, a common type of midcerebellar glioma of childhood. Arch Neurol Psychiat 1925, 14:192–224.

    Article  CAS  Google Scholar 

  25. Cushing H: Experiences with cerebellar medulloblas tomas: Critical review.APMIS1930, 1:1–86.

    Google Scholar 

  26. Rorke LB: The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors.J Neuropathol Exp Neurol1983, 42: 1–15.

    Article  PubMed  CAS  Google Scholar 

  27. Rubinstein LJ: Embryonal central neuroepithelial tumors and their differentiating potential: A cytogenetic view of a complex neuiooncgl6gical problem.J Neurosurg1985,62:795–805.

    Article  PubMed  CAS  Google Scholar 

  28. Albright AL, Wisoff JR, Zeltzer P,et al.: Prognostic factors in children with supratentorial (nonpineal) primitive neuroectodermal tumors.Pediatr Neurosurg1995, 22:1–7.

    Article  PubMed  CAS  Google Scholar 

  29. Pigott TJD, Punt JAG, Lowe JS,et al.: The clinical, radiological and histopathological features of cerebral primitive neuroectodermal tumors.Br J Neurosurg1990,4:287–298.

    Article  PubMed  CAS  Google Scholar 

  30. Kayama T, Yoshimoto T, Shimizu H,et al.: Neonatal medulloblastoma.J Neurooncol1993, 15:157–163.

    Article  PubMed  CAS  Google Scholar 

  31. Meyers SP; Kemp SS, Tarr RW: MR imaging features of medulloblastomas.AJR1992, 158:859–865.

    PubMed  CAS  Google Scholar 

  32. Mueller DP, Moore SA, Sato Y,et al.: MRI spectrum of medulloblastoma.Clin Imaging1992, 16:250–255.

    Article  PubMed  CAS  Google Scholar 

  33. Albright AL, Wisoff JH, Zeltzer PM,et al.: Effects of medulloblastoma resections on outcome in children: A report from the Children's Cancer Group.Neurosurgery1996, 38:264–271.

    Google Scholar 

  34. Abraham J, Chandy J: Ventriculo-atrial shunt in the management of posterior fossa tumors. Preliminary report.J Neurosurg1963, 20:252–253.

    Article  PubMed  CAS  Google Scholar 

  35. Grabb PA, Albright AL, Sclabassi RJ,et al: Conkuous intraoperative electromyography of cranial nerves during resection of fourth ventricular tumors in children.J Neurosurg1997, 86:1–4

    Article  PubMed  CAS  Google Scholar 

  36. Tomita T: Medulloblastomas. InNeurological Surgery. Edited by Youmans JR, edn 4. Philadelphia: WB Saunders; 1996:2570–2592.

    Google Scholar 

  37. Park TS; Hoffman HJ, Hendrick EB,et al.: Medulloblastoma: Clinical presentation and management. Experience at the Hospital for Sick Children, Toronto, 1950–1980.J Neurosurg1983, 58:543–552.

    Google Scholar 

  38. Hoffman H, Hendrick E, Humphreys R: Metastasis via ventriculoperitoneal shunt in patients with medulloblastoma. J Neurosurg 1976, 44:562–566.

    Article  PubMed  CAS  Google Scholar 

  39. Berger MS, Baumeister B, Geyer JR,et al.: The risks of metastases from shunting in children with primary central nervous system tumors.J Neurosurg1991, 74:872–877.

    Article  PubMed  CAS  Google Scholar 

  40. Sutton LN, Phillips PC, Molloy PT: Surgical management of medulloblastoma.J Neurooncol1996, 29:9–21.

    Article  PubMed  CAS  Google Scholar 

  41. Cochrane DD, Gustavsson B, Poskitt KP,et al: The surgical and natural morbidity of aggressive resection for posterior fossa tumors in childhood. Pediatr Neurosurg 1994, 20:19–29.

    Article  PubMed  CAS  Google Scholar 

  42. Wisoff JH, Epstein FJ: Pseudobulbar palsy after posterior fossa operation in children.Neurosurgery1984, 15:707–709.

    Article  PubMed  CAS  Google Scholar 

  43. Pollack IF, Polinko P, Albright AL,et al: Mutism and pseudobulbar symptoms after resection of posterior fossa tumors in children: Incidence and pathophysiology.Neurosurgery 1995, 37:885–893.

    Article  PubMed  CAS  Google Scholar 

  44. Pollack IF, Polinko P, Albright AL,et al.: Neurobehavioral syndromes following posterior fossa surgery in children. InThe Cerebellum and Cognition. Edited by Schmahmann JD. San Diego: Academic Press; 1997.

    Google Scholar 

  45. Chang CH, Housepian EM, Herbert C Jr: An operative staging system and megavoltage radiotherapeutic technique for cerebellar medulloblastoma.Radiology1969, 93:1351–1359.

    PubMed  CAS  Google Scholar 

  46. Evans AE, Jenkin DT, Sposto R,et al.: The treatment of medulloblastoma. Results of a prospective randomized trial of radiation therapy with and without CCNU, vincristine, and prednisone.J Neurosurg1990, 72:572–582.

    Article  PubMed  CAS  Google Scholar 

  47. Tait DM, Thornton-Jones H, Bloom HJG,et al.: Adjuvant chemotherapy for medulloblastoma: The first multi-centre control,.trial of the International Society of Paediatric Oncology (SIOP I).Bur J Cancer1990, 26:464–469.

    CAS  Google Scholar 

  48. Bourne JP, Geyer R, Berger M,et al: The prognostic significance of postoperative residual contrast enhancement on CT scan in pediatric patients with medulloblastoma.J Neurooncol1992, 14:262–270.

    Article  Google Scholar 

  49. Jenkin D, Goddard K, Armstrong D,et al: Posterior fossa medulloblastoma in childhood: Treatment results and a proposal for a new staging system.Int J Radiat Oncol Biol Phys1990,19:265–274.

    PubMed  CAS  Google Scholar 

  50. Krischer JP, Ragab AR, Kun L,et al.: Nitrogen mustard, vincristine, procarbazine, and prednisone as adjuvant chemotherapy in the treatment of medulloblastoma.J Neurosurg1991,74:905–909.

    Article  PubMed  CAS  Google Scholar 

  51. Patterson R, Farr RF: Cerebellar medulloblastomas: treatment by irradiation of the whole central nervous system. Acta Radiol1953, 39:323–336.

    Google Scholar 

  52. Kun LE, Constine LS: Medulloblastoma: Caution regarding new treatment approaches.Int J Radiat Oncol Biol Phys1991,20:897–899.

    Article  PubMed  CAS  Google Scholar 

  53. Deutsch M, Thomas PRM, Krischer J,et al.: Results of a prospective randomized trial comparing standard dose neuraxis irradiation (3,600 cGy/20) with reduced neuraxis irradiation (2,340/13) in patients with low-stage medulloblastoma: A combined Children's Cancer Group Pediatric Oncology Group study.Pediatr Neurosurg1996, 24:167–177.

    Article  PubMed  CAS  Google Scholar 

  54. Packer RJ, Sutton LN, Goldwein JW,et al.: Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma.J Neurosurg1991, 74:433–440.

    Article  PubMed  CAS  Google Scholar 

  55. Packer RJ, Sutton LN, Elterman R,et al.: Outcome for children with medulloblastoma treated with radiation and cisplatin, CCNU, and vincristine chemotherapy.J Neurosurg1994,81: 690–698.

    Article  PubMed  CAS  Google Scholar 

  56. Halberg FE, Wara WM, Fippin LF,et al.: Low-dose craniospinal radiation therapy for medulloblastoma.Int J Radiat Oncol Biol Phys1991, 20:651–654.

    Article  PubMed  CAS  Google Scholar 

  57. Bailey CC, Gnekow A, Wellek S,et al: Prospective randomized trial of chemotherapy given before radiotherapy in childhood medulloblastoma. International Society of Paediatric OncQlogy (SIOP) and the German Society of Paediatric Oncology(GPO):SIOP II.Med Pediatr Oncol 1995,25:166–178.

    Article  PubMed  CAS  Google Scholar 

  58. Packer RJ, Goldwein J, Nicholson S,et al: Reduced-dose radiotherapy plus chemotherapy for medulloblastoma. Proceedings of the Northern American Society for Neurooncology Meeting 1996[abstract 97]. Sante Fe: The Northern American Society for Neuro-oncology Meeting; 1996.

    Google Scholar 

  59. Pendergrass TW, Milstein JM, Geyer JR,et al.: Eight drugs in one day chemotherapy for brain tumors: Experience in 107 children and rationale for preradiation chemotherapy. J Clin Oncol1987, 5:1221–1231.

    PubMed  CAS  Google Scholar 

  60. Gentet JC, Bouffet E, Doz F,et al.: Preirradiation chemotherapy including" eight drugs in one day" regimen and high-dose methotrexate in childhood medulloblastoma: Results of the M7 French Cooperative Study. J Neurosurg 1995, 82:60–14.

    Google Scholar 

  61. Duffner PK, Horowitz ME, Krischer JP,et al: Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors.N Engl J Med1993,24:1725–1731.

    Article  Google Scholar 

  62. Kovnar EH, Kellie SJ, Horowitz ME,et al: Preirradiation cisplatin and etoposide in the treatment of high-risk medulloblastoma and other malignant embryonal tumors of the central nervous system: A phase II study.J Clin Oncol1990, 8:330–336.

    PubMed  CAS  Google Scholar 

  63. Belza MG, Donaldson SS, Steinberg GK,et al.: Medulloblastoma: Freedom from relapselonger than 8 years: A therapeutic cure?J Neurosurg1991, 75:575–582.

    Article  PubMed  CAS  Google Scholar 

  64. Torres CF, Rebsamen S, Silber JH,et al.: Surveillance scanning of children with medulloblastoma.N Engl J Med 1994, 330:892–895.

    Article  PubMed  CAS  Google Scholar 

  65. Mendel E, Levy ML, Raffel C,et al.: Surveillance imaging in children with primitive neuroectodermal tumors.Neurosurgery 1996,38:692–695.

    Article  PubMed  CAS  Google Scholar 

  66. Friedman HS, Mahaley MS, Schold SC,et al.: Efficacy of vincristine and cyclophosphamide in the therapy of recurrent medulloblastoma.Neurosurgery1986, 18:335–340.

    Article  PubMed  CAS  Google Scholar 

  67. Bertolone SJ, Baum ES, Krivit W,et al.: A phase II study of cisplatin therapy in recurrent childhood brain tumors: A report from the Childrens's Cancer Group.J Neurooncol 1989,7:5–11.

    Article  PubMed  CAS  Google Scholar 

  68. Patrice SJ, Tarbell NJ, Goumnerova LC,et al.: Results of radiosurgery in the management of recUrrent and residual medulloblastoma.Pediatr Neurosurg1995, 22:197–203.

    Article  PubMed  CAS  Google Scholar 

  69. Lundberg JH, Weissman DE, Beatty PA,et al: Treatment of recurrent metastatic medulloblastoma with intensive chemotherapy and allogeneic bone marrow transplantation. I Neuroonco11992, 13:151–155.

    Google Scholar 

  70. Dunkel IJ, Finlay JL: High dose chemotherapy with autologous stem cell rescue for patients with medulloblastoma. J Neurooncol1996, 29:69–74.

    Article  PubMed  CAS  Google Scholar 

  71. Brown WD, Tavare CJ, Sobel EL,et al.: Medulloblastoma and Collins' Law: A critical review of the concept of a period of risk for tumor recurrence and patient survival. Neurosurgery1995,36:691–697.

    Article  PubMed  CAS  Google Scholar 

  72. Lefkowitz IB, Packer RJ, Ryan SG,et al.: Late recurrence of primitive neuroectodermal tumor/medulloblastoma. Cancer 1988, 62:826–830.

    CAS  Google Scholar 

  73. Ellenberg L, McComb JG, Siegel S,et al.: Factors affecting intellectual outcome in pediatric brain tumor patients. Neurosurgery1987,21:638–644.

    Article  PubMed  CAS  Google Scholar 

  74. Radcliffe J, Packer RJ, Atkins TE,et al.: Three and fouryear cognitive outcome in children with noncortical brain tumors treated with whole-brain radiotherapy.Ann Neurol1992,32:551–554.

    Article  PubMed  CAS  Google Scholar 

  75. Duffner PK, Cohen ME, Thomas PRM,et al.: The longterm effects of cranial irradiation on the central nervous system.Cancer1985,56:1841–1846.

    Article  PubMed  CAS  Google Scholar 

  76. Johnson DL, McCabe MA, Nicholson HS,et al: Quality of long-term survival in young children with medulloblastoma,J Neurosurg1994, 80: 1004–10 10.

    Article  PubMed  CAS  Google Scholar 

  77. Hirsch JF, Renier D, Czernichow P,et al: Medulloblastoma in childhood: Survival and functional results.Acta Neurochir1979,48:1–15.

    Article  CAS  Google Scholar 

  78. Seaver E, Geyer R, Sulzbacher S,et al: Psychosocial adjustment in long-term survivors of childhood medulloblastoma and ependymoma treated with craniospinal irradiation.Pediatr Neurosurg1994, 20:248–253.

    Article  PubMed  CAS  Google Scholar 

  79. Hoppe-Hirsch E, Renier D, Lellouch-Tubiana A,et al.: Medulloblastoma in childhood: Progressive intellectual deterioration.Child's Nero Syst1990, 6:60–65.

    Article  CAS  Google Scholar 

  80. Kanev PM, Lefebvre JF, Mauseth RS,et al.: Growth hormone deficiency following radiation therapy of primary brain tumors in children.J Neurosurg1991, 74:743–748.

    Article  PubMed  CAS  Google Scholar 

  81. Duffner PK, Cohen ME, Voorhess ML,et al.: Long-term effects of cranial irradiation on endocrine function in children with brain tumors: A prospective study.Cancer 1985, 56:2189–193.

    Article  PubMed  CAS  Google Scholar 

  82. Livesey EA, Hiridmarsh PC, Brook CGD,et al.: Endocrine disorders following treatment of childhood brain tumours.Br J Cancer1990,61:622–625.

    Article  PubMed  CAS  Google Scholar 

  83. Probert JC, Parker BR, Kaplan HS: Growth retardation in children after megavoltage irradiation of the spine. Cancer1973, 32:634–639.

    Article  PubMed  CAS  Google Scholar 

  84. Clayton PE, Shalet SM, Price DA,et al.: Testicular damage after chemotherapy for childhood brain tumors.J Pediatr 1988, 112:922–926.

    Article  PubMed  CAS  Google Scholar 

  85. Hawkins MM, Draper GJ, Kingston IE: Incidence of second primary tumors among childhood cancer survivors. Br J Cancer1987,56:339–347.

    Article  PubMed  CAS  Google Scholar 

  86. Farwell J, Flannery JT: Second primaries in children with central nervous system tumors.J Neurooncol1984, 2:371–375.

    Article  PubMed  CAS  Google Scholar 

  87. De Vathaire F, Francois P, Hill C,et al: Role of radiotherapy and chemotherapy in the risk of second malignant neoplasms after cancer in childhood.Br J Cancer1989, 59:792–796.

    Article  PubMed  Google Scholar 

Download references

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 1998 Current Medicine, Inc.

About this chapter

Cite this chapter

Follack, I.F. (1998). Current Management of Medulloblastoma. In: Salcman, M. (eds) Current Techniques in Neurosurgery. Springer, New York, NY. https://doi.org/10.1007/978-1-4613-9350-4_19

Download citation

  • DOI: https://doi.org/10.1007/978-1-4613-9350-4_19

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4613-9352-8

  • Online ISBN: 978-1-4613-9350-4

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics